Language deficits in pre-symptomatic Huntington's disease: evidence from Hungarian.
Language deficits in pre-symptomatic Huntington's disease: evidence from Hungarian.
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DOI：
10.1016/j.bandl.2012.04.001
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发表时间：
2012-06
影响因子：
2.5
通讯作者：
Ullman, Michael T.
中科院分区：
文献类型：
--
作者：
Nemeth, Dezso;Dye, Cristina D.;Sefcsik, Tamas;Janacsek, Karolina;Turi, Zsolt;Londe, Zsuzsa;Klivenyi, Peter;Kincses, Zsigmond Tamas;Szabo, Nikoletta;Vecsei, Laszlo;Ullman, Michael T.
关键词：
少数研究调查了亨廷顿病（HD）中的语言。这些研究普遍报道了语言规则（语法）方面的异常，包括句法和词法。英语和法语动词屈折形态学的几项研究报告了过度活跃的规则处理的证据，例如过度后缀错误（例如，Walkeded）和过度正则化（例如，挖）。在这里，我们扩大调查匈牙利语，芬兰乌戈尔语的黏着语与复杂的形态，名词屈折，并遗传学证明症状前的亨廷顿氏病（前HD）。虽然HD前期患者没有临床、运动或认知症状，但潜在的病理可能已经开始，因此敏感的行为测量可能会揭示已经存在的损伤。事实上，在匈牙利形态生成任务中，HD前患者出现了过度后缀化和过度正则化错误。研究结果表明，过度活跃的规则处理的一般性，在HD和前HD，跨语言从不同的家庭与不同的形态系统，并为动词和名词的变形。由于HD前的神经病理学似乎在很大程度上限于尾状核和相关结构，研究结果进一步牵连这些结构的语言，特别是在规则处理。最后，对HD有效治疗的需求，这可能部分取决于灵敏地测量疾病早期变化的能力，表明屈折形态学和其他语言测量可能为评估和治疗HD前和HD早期变性提供有用的诊断、跟踪和治疗工具。
A limited number of studies have investigated language in Huntington's disease (HD). These have generally reported abnormalities in rule-governed (grammatical) aspects of language, in both syntax and morphology. Several studies of verbal inflectional morphology in English and French have reported evidence of over-active rule processing, such as over-suffixation errors (e.g., walkeded) and over-regularizations (e.g., digged). Here we extend the investigation to noun inflection in Hungarian, a Finno-Ugric agglutinative language with complex morphology, and to genetically proven pre-symptomatic Huntington's disease (pre-HD). Although individuals with pre-HD have no clinical, motor or cognitive symptoms, the underlying pathology may already have begun, and thus sensitive behavioral measures might reveal already-present impairments. Indeed, in a Hungarian morphology production task, pre-HD patients made both over-suffixation and over-regularization errors. The findings suggest the generality of over-active rule processing in both HD and pre-HD, across languages from different families with different morphological systems, and for both verbal and noun inflection. Because the neuropathology in pre-HD appears to be largely restricted to the caudate nucleus and related structures, the findings further implicate these structures in language, and in rule-processing in particular. Finally, the need for effective treatments in HD, which will likely depend in part on the ability to sensitively measure early changes in the disease, suggests the possibility that inflectional morphology, and perhaps other language measures, may provide useful diagnostic, tracking, and therapeutic tools for assessing and treating early degeneration in pre-HD and HD.
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影响因子：
11
作者：
Kipps, CM;Duggins, AJ;McCusker, EA
通讯作者：
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影响因子：
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影响因子：
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作者：
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通讯作者：
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